Evaluation Of Congenital Heart Disease Research Articles

The New Frontiers of Fetal Imaging: MRI Insights into Cardiovascular and Thoracic Structures.

Fetal magnetic resonance imaging (fMRI) represents a second-line imaging modality that provides multiparametric and multiplanar views that are crucial for confirming diagnoses, detecting associated pathologies, and resolving inconclusive ultrasound findings. The introduction of high-field magnets and new imaging sequences has expanded MRI's role in pregnancy management. Recent innovations in ECG-gating techniques have revolutionized the prenatal evaluation of congenital heart disease by synchronizing imaging with the fetal heartbeat, thus addressing traditional challenges in cardiac imaging. Fetal cardiac MRI (fCMR) is particularly valuable for assessing congenital heart diseases, especially when ultrasound is limited by poor imaging conditions. fCMR allows for detailed anatomical and functional evaluation of the heart and great vessels and is also useful for diagnosing additional anomalies and analyzing blood flow patterns, which can aid in understanding abnormal fetal brain growth and placental perfusion. This review emphasizes fMRI's potential in evaluating cardiac and thoracic structures, including various gating techniques like metric optimized gating, self-gating, and Doppler ultrasound gating. The review also covers the use of static and cine images for structural and functional assessments and discusses advanced techniques like 4D-flow MRI and T1 or T2 mapping for comprehensive flow quantification and tissue characterization.

Cornelia De Lange Syndrome with left heart hypoplasia: a case study

Cornelia de Lange syndrome (CdLS) is characterized by slow growth that can lead to short stature. Despite mutations in the NIPBL, RAD21 or SMC3 genes, CdLS is thought to be inherited in an autosomal dominant manner. Findings include intellectual disability and bone abnormalities in the upper extremities and distinct facial features. It has similar characteristics to autism spectrum disorder, which is a developmental condition that affects communication and social interaction. The physical and clinical findings of a one-and-a-half-year-old girl patient diagnosed with CdLS were reported. In addition to NIPBL gene mutation, atrioventricular septal defect (AVSD), an overriding aorta, and a hypoplastic left heart (HLH) were observed in our case. This study revealed the patient's physical and clinical findings consistent with the diagnosis of CdLS. Additionally emphasized HLH. The importance of performing corrective surgery immediately following the evaluation of congenital heart diseases in CdLS patients can be emphasized.

More effective diagnostic methods for the early evaluation of congenital heart disease: A systematic review

Objectives: To evaluate which diagnostic methods are most effective for the early assessment of possible cardiac anomalies in utero and in the neonatal period, with the aim of analyzing the impact of tests performed during prenatal care and correlating early diagnosis with the risk of infant mortality. Method: a systematic review was carried out using the Science and Pubmed platforms in the period between 2012 and 2022, where 'newborns' OR 'premature infants' AND 'congenital heart disease' OR 'congenital heart defects' OR 'congenital heart defects' were used in the search” OR “baby's heart” AND “early diagnosis” OR “prenatal diagnosis”. Successively, the articles found were selected according to the previously determined inclusion and exclusion criteria to suit the type of research. Results: after analyzing 97 articles, 23 were in the same parameters and were considered in the study, addressing exams such as ultrasound, nuchal translucency, fetal echocardiogram, genetic tests, pulse oximetry, cardiovascular physical examination and echocardiographic reassessment. Conclusion: given the assumption, it was found that prior identification of the pathological change results in a more improved and prepared therapeutic plan and better psychological acceptance by parents. Therefore, the importance of disseminating the various methods available and how to perform them correctly is justified in order to make an early diagnosis of congenital heart diseases and contribute to reducing the mortality of these newborns.

The Role of CT Angiography Above Echocardiography in Evaluations of Congenital Heart Disease in TCH (2013-2018)

Back ground: Congenital heart disease (CHD) one of the most commonly occurring congenital anomaly, affecting 4-10/1000 live births, Echocardiography is the mainstay of diagnosis, the recent developments in CT techniques increasing role of CT in the evaluation of congenital heart disease that minimize the radiation exposure particularly for children. Objective: This study is performed to assess and high light the role of multi-detector computed tomography (CT angiography) in the evaluation of cardiovascular abnormalities as (Cono- truncal defects) in children and the Clinical indications above trans-thoracic echocardiography. Method: This study included 70 patients, they were referred or following in pediatric cardiology clinic at TCH from January 2013 till October 2018. All patients presented with history or clinical suspicion of Cono- truncal defect were included in the study, for all patient's full history and clinical examination were obtained. An echocardiography examination was done. CT angiography was performed by Philips machine (16 segment). Results: Pulmonary anomalies represent the majority of detected congenital defects in our study, represent 37 cases (53 %) and the commonest pulmonary anomaly is pulmonary stenosis. The majority of patient shows the same finding in echocardiography & CT angiography which represent 47 cases (67.1%), while in 12 cases (17%) CT angiography add more details in regards to the size of the great vessels and the peripheral structure (peripheral pulmonary arteries), while CT angiography rule-out the echocardiography finding in only 11 cases (15 %). Out of 23 cases of patients with Aortic anomaly, 19 cases (82.6%) have the same finding in echocardiography while in 3 cases (13%) the result of CT rolled out the echo finding, and 29 (78.4%) cases out of 37 cases with pulmonary anomalies have same finding in both echocardiography and CT angiography. Conclusion: We conclude that CT angiography is considered a non-invasive diagnostic tool complementary to the cardiac echocardiography in the diagnosis of complex heart defects especially in pulmonary anomalies. Recommendation: Similar study need to be conducted with large sample to confirm the extent of this finding.

COMPARISON BETWEEN ECHOCARDIOGRAPHY AND CARDIAC COMPUTED TOMOGRAPHY FOR THE DIAGNOSIS OF CONGENITAL HEART DISEASES

Congenital heart disease (CHD) is a significant cause of morbidity and mortality worldwide. Accurate diagnosis is crucial for appropriate management and intervention. Echocardiography and cardiac computed tomography (CT) are commonly used for diagnosing CHD, yet their diagnostic reliability in a specific clinical setting requires evaluation. The objective of the study was to assess the diagnostic reliability of echocardiography and cardiac computed tomography (CT) for congenital heart disease (CHD) at Jinnah Hospital in Lahore. A cross-sectional study was conducted at the Department of Radiology, Chaudhary Pervez Ellahi Institute of Cardiology, from January 2022 to December 2022. One hundred patients suspected of congenital cardiac disorders were enrolled, spanning various age groups. Data were collected retrospectively, including procedure notes, CT scan results, echocardiograms, and demographic information. Descriptive statistics were used to analyse the data, with median and percentiles calculated using SPSS. A significance level of p < 0.05 was considered statistically significant. Of the 100 patients included in the study, 69 were men, and 31 were women, with ages ranging from a few days to eighteen years. Two categories of malformations were identified: intracardiac and extracardiac. Using surgical and conventional angiography results as the gold standard, 544 anomalies were confirmed and analysed, including 46 extracardiac and 196 intracardiac findings. Echocardiography demonstrated an overall diagnostic accuracy of 96.86%, while cardiac CT exhibited a diagnostic accuracy of 98.27% for cardiac congenital abnormalities. Based on the results, the cardiac computed tomography (CT) provides superior visualization of extracardiac abnormalities, with a diagnostic accuracy of 99.02%. In contrast, echocardiography remains the preferred modality for detecting intracardiac anomalies, boasting a diagnostic accuracy of 99.31%. The complementary use of echocardiography and cardiac CT enhances the assessment and evaluation of congenital heart diseases, facilitating surgical planning and intervention.

Comparison of Cardiac Computed Tomography and Echocardiography for the Diagnosis of Congenital Heart Diseases

Objectives: To compare the diagnostic accuracy of cardiac Computed Tomography (CT) and echocardiography for the diagnosis of congenital heart diseases (CHD) at Rehman Medical Institute (RMI), Peshawar. Methodology: We conducted a cross sectional study at Rehman Medical Institute between January 2020 to July 2022. Patients of different ages referred for suspicion of congenital heart diseases were enrolled. The data was collected prospectively which included demographics, Echocardiography findings, CT scan results and procedure notes. Data was analyzed on SPSS the results of descriptive variables expressed as median and percentile. A p-value <0.05 was considered significant. Results: We included 129 patients, with 73 male and 56 females having age from few days to 18 years. The malformations were divided into intra and extra cardiac groups. 665 malformations were confirmed and analyzed keeping surgical and conventional angiography findings as gold standard. Among these 283 were intra and 99 extra cardiac findings. The echo and CT scan missed 2 and 4 intracardiac anomalies respectively. In case of extracardiac anomalies echo missed 14 anomalies however CT correctly diagnosed all. Both intra and extracardiac results are significant with a p value <0.05. Conclusion: We conclude that Echocardiography is preferred modality for intracardiac anomalies evident by 99.3% diagnostic accuracy while CTA provides better demonstration of extra cardiac abnormalities having diagnostic accuracy of 99.9%. Echocardiography and CTA are both complementary for assessment and evaluation of congenital heart diseases acting as guide to surgery.

Evaluation of cardiovascular morphology and associated anomalies in patients with crossed pulmonary arteries on multidetector computed tomography angiography.

The present study sought to study morphology and associated cardiovascular anomalies in patients with crossed pulmonary arteries on multidetector computed tomography (CT) angiography. We retrospectively evaluated all CT angiography studies performed at a tertiary medical center from January2014 to December2021 to identify patients with crossed pulmonary arteries. The associated cardiovascular anomalies as identified on CT angiography were evaluated. Out of 4773 patients who had undergone CT angiography for evaluation of congenital heart diseases, we identified 24 (0.5%) patients (18 males; mean age: 7.7 years) with crossed pulmonary arteries. Tetralogy of Fallot was associated in seven (29.17%) patients, double outlet right ventricle in one (4.17%) patient, and common arterial trunk in four (16.67%) patients. An aortopulmonary window was seen in three (12.5%) patients while atrial septal defect and ventricular septal defect were seen in two (8.33%) and 16 (66.67%) patients, respectively. Aortic arch anomalies were present in 16 (66.67%) patients including interrupted aortic arch and coarctation of aorta with hypoplastic aortic arch seen in two (8.33%) patients each. A double aortic arch with an atretic left arch was seen in one (4.17%) patient. Coronary artery anomalies were seen in three (12.5%) patients. Crossed pulmonary arteries are a rare anomaly and their presence suggests the coexistence of a variety of cardiovascular anomalies, including aortic arch anomalies and outflow tract malformations. Comprehensive CT angiography-based evaluation of cardiovascular morphology is imperative in the presence of crossed pulmonary arteries to facilitate presurgical planning.

Speckle tracking echocardiography strain analysis for the assessment and monitoring of myocardial mechanics after pediatric cardiac surgery

Abstract Funding Acknowledgements Type of funding sources: None. Background. Speckle-tracking echocardiography (STE) is a relatively new ultrasound technique that exploits acoustic reflections and artifacts within the ultrasonic window, with the aim of analyzing global myocardial deformation and segmenting. STE has proved to be more sensitive in identifying a myocardial dysfunction so gained increasing value in the evaluation of congenital heart diseases (CHD) even though its use, in pediatric cardiac surgery, is still limited. The present study aims to evaluate the global and left ventricular (LV) systolic impairment after pediatric cardiac surgery by STE strain (ε) analysis. Methods We prospectively enrolled 120 children undergoing cardiac surgery for CHD. Echocardiography was performed at four different times: pre-operatively, 12-36 hours (Time-1), 3-5 days (Time-2), 6-8 days (Time-3). Images were obtained in the 4-2-and 3 apical chamber’s views to derive LV global and regional (basal/mid/apical) ε values. Results. We collected data from 332 examinations in 120 children (mean age: 2.33 ±3.91, range: 0-16 years) at different post-operative times. 120 age-matched healthy children (3.1 ± 4.2 years) served as controls. All global, basal, and mid LVε values reduced after surgery; the lowest values were at Time-1 (p < 0.0001) but increased thereafter. At discharge, all global, basal, and mid LVε values persisted lower than pre-operatively and healthy children (p < 0.05). Instead, apical segments (lowest at baseline), increased after surgery (p < 0.0001), but remained lower compared to controls. LV ejection fraction (LVEF) decreased at Time-1 (p = 0.0003) but promptly recovered to Time-2, to standardize at Time-3. Conclusions. Despite LVEF was normalized by the discharge, STE ε has shown a systolic impairment after cardiac surgery that disclosed a gradual improvement but still uncomplete normalization at discharge. Regional STE differences revealed a discrepancy base-apex: apical segments, contrary to all the other regions, showed hypercontractility after surgery. The slower recovery of LVε values compared to LVEF, suggests that STE ε analysis may be more accurate in the follow-up of mild LV post-surgical impairment. Abstract Figure. LV ventricular ε values

Determination of Sensitivity and Specificity of High Resolution CT Scan in Evaluation of Cardiac and Extra-Cardiac Anomalies among Bangladeshi Children

Congenital heart disease (CHD) is one of the important causes of morbidity and mortality in the pediatric age group. Invasive angiography is considered as gold standard method for the diagnosis of pediatric cardiovascular diseases. Multi-detector CT cardiac angiogram by using 64 slice scanner is a useful modality and suitable alternative to previous invasive procedure in evaluation of congenital heart disease. The present study was conducted in the Department of Cardiology of NICVD during the period of September 2019 to August 2020 (One year). We selected 40 patients presenting in the outpatient department of National Institute of Cardiovascular Diseases and Hospital (NICVD) who were diagnosed to have congenital heart disease clinically and radiologically. CT angiogram revealed ASD in 10%, coarctation of aorta in 5%, Pentalogy of Fallot in 2.5%, Tetralogy of Fallot (TOF) in 32.5%, Ventricular septal defect (VSD) in 30.0%, PAH in 2.5%, PS in 5%, RVH with PS in 2.5%, PDA in 5% and Tricuspid atresia with ASD with VSD with PS in 2.5% patients. Conventional catheter angiogram revealed ASD in 5%, VSD in 32.5%, TOF in 30%, PS in 12.5%, Coarctation of aorta in 5% and Pentalogy of Fallots in 5%. The diagnostic performance of MDCT in the diagnosis and evaluation of congenital heart disease was high.

Anomalous subaortic course of brachiocephalic vein: Evaluation on multidetector computed tomography angiography

To evaluate the morphology and associated cardiovascular abnormalities in patients with an anomalous subaortic course of brachiocephalic vein on multidetector computed tomography (CT) angiography. A retrospective study was performed at a tertiary referral institute to identify patients with subaortic brachiocephalic vein on multidetector CT (MDCT) angiography using dual source CT scanner between January 2014 and July 2021. The morphology of the subaortic brachiocephalic vein along with the cardiovascular anatomy and associated anomalies were evaluated. Out of 4349 patients who had undergoneMDCT angiography for evaluation of congenital heart diseases, we identified 126 (2.9%) patients with subaortic brachiocephalic vein. The subaortic brachiocephalic vein was left-sided in 125 patients while a right-sided subaortic brachiocephalic vein was identified in a patient with left isomerism. Common cardiovascular associations included tetralogy of Fallot (109/126; 88.1%), double outlet right ventricle (8/126; 6.3%) and common arterial trunk (5/126; 3.9%). The presence of a right aortic arch was seen in 78/126 (62%) patients. Some degree of right ventricular outflow obstruction was present in 119/126 (94.4%) patients; pulmonary stenosis was seen in 78 (62%) patients while pulmonary atresia was seen in 41 (32.5%) patients. A subaortic brachiocephalic vein can coexist with various complex congenital heart diseases, most commonly tetralogy of Fallot and commonly associated with right aortic arch and pulmonary stenosis/atresia. It is important to identify this anomalous course of brachiocephalic vein before performing surgical procedures or venous catheterization to avoid potential complications.

Left Ventricular Systolic Impairment after Pediatric Cardiac Surgery Assessed by STE Analysis.

Background: Speckle-tracking echocardiography (STE) has gained increasing value in the evaluation of congenital heart diseases (CHD); however, its use in pediatric cardiac surgery is limited. Aim: To evaluate left ventricular (LV) systolic impairment after biventricular pediatric cardiac surgery by STE strain (ε) analysis. Methods: We prospectively enrolled 117 children undergoing cardiac surgery for CHD. Echocardiography was performed at four different times: pre-operatively, 12–36 h (Time 1), 3–5 days (Time 2), and 6–8 days (Time 3). Images were obtained in the 4-2-and 3 apical chamber’s views to derive LV global and regional (basal/mid/apical) ε values. Results: At different postoperative times, we performed 320 examinations in 117 children (mean age: 2.4 ± 3.9, range: 0–16 years); 117 age-matched healthy children served as controls. All global, basal, and mid LVε values decreased after surgery; the lowest values being at Time 1 (p < 0.0001), which increased thereafter. At discharge, all global, basal, and mid LVε values remained lower than in pre-operative and healthy children (p < 0.05). Instead, apical segments (lowest at baseline) increased after surgery (p < 0.0001) but remained lower compared to controls. LV ejection fraction (LVEF) decreased at Time 1 (p = 0.0004) but promptly recovered to Time 2 and normalized at Time 3. Conclusions: STE ε analysis revealed a significant LV systolic impairment after surgery with amelioration thereafter but incomplete normalization at discharge. Base-apex differences emerged with apical segments that, contrary to all the other regions, showed relative hypercontractility after surgery. The slower recovery of LVε values compared to LVEF suggests that STE ε analysis may be more accurate for the follow-up of mild LV post-surgical impairment.

Dual-source and prospective gated low dose neonatal cardiac computed tomography in evaluation of congenital heart disease

BackgroundThere are very limited studies describing radiation dose using prospective gated dual-source Cardiac CT in neonates. ObjectivesThe aim of this study is to describe the radiation exposure parameters in newborns using modern fast CT scanner (prospective gated dual-source technique) and the role of CT scan in providing useful information for optimal management planning. MethodsRetrospective review of 60 neonates who underwent cardiac CT at our Children's Hospital between November 2019 to November 2020 using the Siemens Somatom Force Dual-Source scanner (Siemens Medical Solutions, Malvern, PA, USA) and prospective gated technique. Patient demographic information, total dose length product (DLP), computed tomographic dose index volume (CTDIvol), patient average heart rate during the scan, indication for the study and patient diagnosis were reviewed. The estimated effective radiation dose was calculated using a previously published conversion rate. Both quantitative and qualitative image quality was evaluated at satellite workstation. ResultsMedian effective radiation dose for the computed tomographic angiography (CTA) was 0.31 mSv. The median total DLP was 8 mGycm and median CTDIvol was 2.46 mGy. The average image noise was 23 with calculated average signal to noise ratio of 22 and average contrast to noise ratio of 19. The qualitative image quality was excellent or good in all of our study subjects with no repeat CT scan needed due to image quality. ConclusionUsing newer generation dual-source fast scanners with prospective ECG-triggered technique, cardiac CT can be done with very low dose in neonates. Cardiac CT provides better understanding of complex congenital heart disease for optimal management planning when echocardiography does not answer the clinical question.

Anomalous origin of left main coronary artery from pulmonary artery: Patient characteristics and imaging associations on multidetector computed tomography angiography.

To evaluate the various imaging features and associations on multidetector computed tomography (CT) angiography in patients with anomalous origin of left main coronary artery (LMCA) from pulmonary artery (ALCAPA). We retrospectively reviewed multidetector CT angiography studies done for the evaluation of congenital heart diseases at our institution through 2014 to 2021. Cases with ALCAPA were identified and relevant history and imaging findings including the origin of coronary arteries, left ventricular (LV) morphology and functions, intercoronary collaterals, and associated abnormalities were evaluated. Twelve patients (eight males,three adults, and nine children; age range: 2 months to 54 years) with ALCAPA were included. Gradually progressive dyspnea and failure to thrive (6/9; 66.67% each) were the most common symptoms amongchildren, whereas adults were commonly asymptomatic (2/3; 66.67%). The LMCA was originating from pulmonary sinus, main, and right pulmonary artery in 6 (50%), 5 (41.66%), and 1 (8.3%) patients, respectively. In adult-type ALCAPA, right coronary artery (RCA), left anterior descending artery (LAD), and left circumflex artery (LCx) were dilated and tortuous, with the presence of well-developed intercoronary collaterals and preserved LV ejection fractions; these features were not seen in patients of infantile ALCAPA. LV dysfunction with global hypokinesia was the most common wall motion abnormality (7/12; 58.33%). Degree of collateralization could be the key factor determining the time of presentation, clinical symptoms, and LV function, thus influencing clinical outcomes. Patients with infantile ALCAPA present with features of heart failure and have dilated and dysfunctional LV consequent to lack of collaterals, unlike adult-type ALCAPA.

Supravalvar aortic stenosis: Imaging characteristics and associations on multidetector computed tomography angiography.

To evaluate the imaging features and associations in patients with supravalvar aortic stenosis on multidetector computed tomography (CT) angiography. We retrospectively reviewed all CT angiography studies performed for evaluation of congenital heart diseases at our institution through the period from January 2014 to June 2020. Cases with supravalvar aortic stenosis were identified and classified as syndromic and nonsyndromic based on history, physical examination, and relevant investigations. The type and extent of vascular involvement and associated cardiovascular abnormalities were characterized. Supravalvar aortic stenosis was identified in 26/3926 (0.66%) patients (22 males and 4 females; Age range: 2 months to 20 years). Discrete stenosis was seen in 14/26 (53.8%) patients, while diffuse involvement of the ascending aorta to varying degrees was seen in the remaining 12 (46.2%) patients. About 15/26 (57.7%) patients had pulmonary involvement at some level, namely,infundibular, valvar, supravalvar, or peripheral pulmonic stenosiswhile 15/26 (57.7%) patients had coronary arterial involvement either in the form of stenosis, occlusion, or ectasia. Aortic valvular abnormality including thickening, partial fusion, and adhesion of leaflet edges to the sinutubular junction causing reduced coronary inflow was seen in 15/26 (57.7%) patients. Associated ventricular septal defect, patent ductus arteriosus, and mitral valvular prolapse were seen in four (15.4%), five (19.2%), and two (7.7%) patients respectively. Supravalvar aortic stenosis is a rare abnormality showing associated pulmonary arterial involvement, coronary arterial involvement, aortic valvular abnormalities, and associated congenital cardiac defects in the majority of cases, which may influence surgical outcomes.

Cardiac computed tomography angiography in the pre-operative assessment of congenital heart disease in Thailand.

IntroductionCardiac computed tomography angiography (CCTA) plays a vital role in clinical practice in evaluating patients with congenital heart disease (CHD) when the information from echocardiography is equivocal.AimTo test the hypothesis that CCTA has significantly value for pre-operative evaluation of congenital heart disease and practicality in the diagnosis and management of congenital heart disease patients at our tertiary care academic hospital.Material and methodsWe studied a total of 78 congenital heart disease patients (median age: 4.5 years) who had undergone CCTA during the period January 2017 to October 2018 at our tertiary care academic hospital.ResultsThe results were classified as diagnostic categories, and the impact of the procedure on strategizing management was analysed. In each group, the CCTA offered an advantage and provided specific clues for surgical or interventional management. In total, the sensitivity (97.5%), specificity (100%), positive predictive value (100%), negative predictive value (99.38%), and accuracy (99.5%) of CCTA, for which the significant findings were confirmed by surgical or cardiac catheterization, were excellent, with average exposure per CCTA study calculated at 1.41 (0.36–3.28) mSv.ConclusionsCCTA is an excellent non-invasive modality for the evaluation of congenital heart disease patients, with an important diagnostic and decision-aiding role.

Surgical management of unroofed coronary sinus syndrome: A 20-year-single-center experience.

Unroofed coronary sinus syndrome (UCSS) is rare and often associated with the left superior vena cava (LSVC). We report our experience of the clinical features, diagnosis, associated anomalies, surgical procedures, and late outcomes of UCSS during a 20-year period. One hundred and fifty-nine patients with UCSS were treated surgically and followed up between May 1998 and May 2019. UCSS was confirmed by preoperative echocardiography or computerized tomography (CT) scan in 97 patients and by the surgeons intraoperatively in 62 cases, and 100 cases were associated with LSVC. Among these patients, eight cases underwent ligation, and the intracardiac tunnel or baffle was reconstructed in 71 cases to ensure drainage of the LSVC into the right atrium (RA), and the extracardiac procedure was to lead the LSVC into the RA in three patients. Coronary sinus opening was diverted to the RA by the atrial septal patch in three cases. Thirteen patients underwent the reroof procedures, and two patients had untreated LSVC. The associated cardiac lesions were corrected concomitantly. There were five in-hospital deaths. Follow-up of 143 patients of early survivors was done, and no deaths were reported. No serious complications were observed, with the exception of avulsion of the internal tunnel patch in one patient. UCSS is often misdiagnosed during preoperative evaluation of congenital heart diseases. Preoperative transthoracic echocardiography remains the most important method in the diagnosis of UCSS. We adopted multiple surgical approaches to manage the different types of UCSS with LSVC with good results.

Isolation of arch vessels: Imaging characteristics and associations on multidetector computed tomography angiography.

To evaluate the imaging characteristics and associations in patients with isolation of arch vessels on multidetector computed tomography angiography (CTA). We retrospectively reviewed all multidetector CTA studies performed for the evaluation of congenital heart diseases (CHDs) at our institution from January 2014 to June 2020. Cases with isolation of arch vessels were identified. The isolated arch artery and its relationship with patent arterial duct, pulmonary artery, and aortic arch were characterized in addition to other associated intra- and extracardiac anomalies. Isolation of arch vessels was seen in 14/3926 (0.36%) patients. Left subclavian artery (SCA) was the commonest isolated arch vessel, involved in 7/14 (50%) cases. Isolation of right SCA, left brachiocephalic artery, and left common carotid artery was seen in 4 (28.6%), 2 (14.3%), and 1 (7.1%) patient, respectively. The isolated arch vessel was seen associated with right aortic arch in 10/14 (71.4%) cases and was on the opposite side of aortic arch in all 14 (100%) patients. Right-sided nonrestrictive patent arterial duct was seen in 3/14 (21.4%) cases, left-sided nonrestrictive patent arterial duct was seen in 1/14 (7.1%) while a left-sided restrictive patent arterial duct was seen in 3/14 (21.4%) cases. Tetralogy of Fallot (ToF) was the commonest associated anomaly seen in 8/14 (57.1%) patients. Isolation of aortic arch branch vessels is rare, seen most commonly associated with ToF. Left SCA is the commonest involved vessel. CTA is useful not only in the diagnosis of isolation of arch vessels, but also in the presence or absence of associated anomalies which may impact the symptomatology, prognosis, and surgical management.

Evaluation of Congenital Heart Diseases in Neonates with Diabetic Mothers Who Referred to Teaching Hospitals in Qom, Iran

Evaluation of Congenital Heart Diseases in Neonates with Diabetic Mothers Who Referred to Teaching Hospitals in Qom, Iran

Agreement Between 64-Slice Multidetector CT Angiography and Transthoracic Echocardiography in Detection of Extracardiac Findings of Congenital Heart Disease.

To determine the degree of agreement between multi-detector computed tomography angiography and transthoracic echocardiography in diagnostic evaluation of congenital heart disease with extracardiac findings. Descriptive cross-sectional study. Radiology Department, Doctors Hospital and Medical Centre, Lahore, from May to November 2016. Patients with clinical suspicion of cardiovascular malformation were included in the study. All the patients had echocardiography and thoracic computed tomography angiography done to diagnose congenital heart disease. Agreement was labelled in true positive and true negative patients. Computed tomography angiography could detect the extracardiac findings of congenital heart disease in 140 (93.3%) patients, while transthoracic echocardiography in 118 (78.7%) patients. Agreement was detected in 108 (72%) patients. Kappa statistics showed poor agreement (kappa = -0.113). Although transthoracic echocardiography is usually the first line of investigation in evaluation of congenital heart disease; it has its limitations for evaluating congenital heart disease with extracardiac findings, due to intrinsic limitation of the imaged field. Computed tomography angiography has better sensitivity in detecting extracardiac findings of congenital heart disease along with better delineation of anatomy.

P3675Prognostic implications of early detection and treatment of left main coronary artery compression by a pulmonary artery among pulmonary arterial hypertension patients

Abstract Introduction Pulmonary artery aneurysm (PAA) is a common condition among pulmonary arterial hypertension (PAH) patients (38%). A pulmonary artery diameter ≥48 mm is an independent risk factor for sudden cardiac death in PAH patients. In the past years, the diagnosis of potentially lethal complications related to PAA is increasing. These complications include pulmonary artery dissection, bronchial compression and left main coronary artery compression (LMCAC). Our aim is to compare the survival and cause of death between PAA patients with a diagnosed and treated LMCAC and the rest of our PAA population. Methods Among our PAH population, we selected those patients who had a PAA diagnosis, defined as main pulmonary artery dilatation &gt;40 mm. All the information was gathered from our institutional database. Patients with a suspected LMCAC based on computed tomography findings were evaluated with coronary angiography and treated surgically or percutaneously when LMCAC was confirmed. Survival from PAH diagnosis was estimated by the Kaplan–Meier method and compared by log-rank test between groups. Results 86 patients in our cohort were diagnosed with a PAA (24% male, 49.8±12.5 years). In 10 (12%) patients, a LMCAC was diagnosed, 9 of them were treated with a percutaneous coronary intervention and the remaining one underwent PAA resection surgery due to severe pulmonary regurgitation. Among patients with LMCAC, only 3 presented chest pain and 2 had signs of ischemia in cardiopulmonary stress testing. In the other 5 patients, LMCAC was suspected after performing a thoracic computed tomography for different reasons: chronic cough in 1 patient, congenital heart disease evaluation in 2 patients and asymptomatic PAA follow-up in 2 patients. None of the patients presented complications secondary to revascularization. There were no differences between patients with and without LMCAC neither in age (49.5±12.9 vs. 49.6±12.4 years; p=0.982) nor in PAH hemodynamic severity (mean pulmonary artery pressure: 63.1±27.6 vs. 56.6±15.0 mmHg; p=0.486). Patients with LMCAC had larger median pulmonary artery diameter (52.5 [47–60.3] vs 43 [46–50] mm; p=0.024). Among LMCAC patients, 1 (10%) died during follow-up due to neoplastic disease. Among patients without LMCAC, 18 (24%) died (5 [6%] sudden cardiac death; 8 [11%] heart failure; 5 [6%] neoplastic or infectious diseases). Despite LMCAC diagnosis, there were no differences between both groups in time from PAH diagnosis to death (log rank test p=0.38). Conclusion LMCAC is a common complication among PAH patients with a PAA. Its early identification and treatment might reduce the rate of sudden cardiac death among PAH patients. Since LMCAC is often asymptomatic regular screening with non-invasive tests should be incorporated to PAH follow-up protocols.